News about our knowledge of the brain and behavior
from Anthony Risser, Ph.D.

Saturday, July 07, 2007

Williams Syndrome

From tomorrow's New York Times Sunday Magazine:

The Gregarious Brain By DAVID DOBBSPublished: July 8, 2007

If a person suffers the small genetic accident that creates Williams syndrome, he’ll live with not only some fairly conventional cognitive deficits, like trouble with space and numbers, but also a strange set of traits that researchers call the Williams social phenotype or, less formally, the “Williams personality”: a love of company and conversation combined, often awkwardly, with a poor understanding of social dynamics and a lack of social inhibition. The combination creates some memorable encounters. Oliver Sacks, the neurologist and author, once watched as a particularly charming 8-year-old Williams girl, who was visiting Sacks at his hotel, took a garrulous detour into a wedding ceremony. “I’m afraid she disrupted the flow of this wedding,” Sacks told me. “She also mistook the bride’s mother for the bride. That was an awkward moment. But it very much pleased the mother.”

Another Williams encounter: The mother of twin Williams boys in their late teens opened her door to find on her stoop a leather-clad biker, motorcycle parked at the curb, asking for her sons. The boys had made the biker’s acquaintance via C.B. radio and invited him to come by, but they forgot to tell Mom. The biker visited for a spell. Fascinated with how the twins talked about their condition, the biker asked them to speak at his motorcycle club’s next meeting. They did. They told the group of the genetic accident underlying Williams, the heart and vascular problems that eventually kill many who have it, their intense enjoyment of talk, music and story, their frustration in trying to make friends, the slights and cruelties they suffered growing up, their difficulty understanding the world. When they finished, most of the bikers were in tears.